摘要
近年来，有关EB病毒（EBV）相关淋巴组织增殖性疾病（LPD）的文章越来越多，EBV＋LPD被使用越来越广。然而，其中所指的含义却不尽相同，由此引发了理解的困惑和交流的障碍。为此，我们通过文献复习和实际病例观察对EBV＋LPD的概念进行梳理和澄清，并阐述我们对EBV＋LPD的理解和认识。总体而言，目前主流观点认为，EBV＋LPD是指EBV感染的一组具有谱系的淋巴组织疾病，其中包括增生性、交界性、肿瘤性疾病。根据这个概念，EBV＋LPD不包括传染性单核细胞增多症（IM）和急性重症EBV感染（EBV＋嗜血细胞综合征、爆发性IM、致死性IM、爆发性EBV＋T－LPD等），也不包括已经明确命名的EBV＋淋巴瘤（如结外NK/T细胞淋巴瘤、侵袭性NK细胞白血病、Burkitt淋巴瘤、霍奇金淋巴瘤等）。EBV＋LPD目前包括：（1）EBV＋B细胞－LPD：淋巴瘤样肉芽肿、EBV＋免疫缺陷相关LPD、慢性活动性EBV感染（CAEBV）－B细胞型、老年性EBV＋B细胞－LPD等。（2）EBV＋T/NK细胞－LPD：CAEBV－T/NK细胞型、种痘样水疱病、蚊叮超敏反应等。根据EBV＋T/NK细胞－LPD的发展进程，通常可分为1级、2级和3级三个级别。第1级为增生性疾病，第2级为交界性疾病，第3级为肿瘤性疾病。EBV＋LPD不同于单纯的增生性疾病（如IM）但又有重叠，也不同于典型的肿瘤性疾病（如NK/T细胞淋巴瘤）但也有重叠。临床上提高警惕，尽早识别与EBV＋LPD相关的严重并发症发生的苗头，从而及时避免严重并发症的发生，以及在并发症发生后积极治疗挽救生命，应作为管控这类疾病更重要的任务。
In recent years, there are increasing articles concerning Epstein-Barr virus associated lymphoprotiferative disorder （EBV ＋ LPD ） , and the name of EBV ＋ LPD is used widely. However, the meaning of EBV ＋ LPD used is not the same, which triggered confusion of the understanding and obstacles of the communication. In order to solve this problem. Literature was reviewed with combination of our cases to clarify the concept of EBV ＋ LPD and to expound our understanding about it. In general, it is currently accepted that EBV ＋ LPD refers to a spectrum of lymphoid tissue diseases with EBV infection, including hyperplasia, borderline lesions, and neoplastic diseases. According to this concept, EBV ＋ LPD should not include infectious mononucleosis （IM） and severe acute EBV infection （EBV ＋ hemophagocytic lymphohistiocytosis, fatal IM, fulminant IM, fulminant T-cell LPD） , and should not include the explicitly named EBV ＋ lymphomas （ such as extranodal NK/T cell lymphoma, aggressive NK cell leukemia, Burkitt lymphoma, and Hodgkin lymphoma, etc. ） either. EBV ＋ LPD should currently include： （ 1 ） EBV ＋ B cell-LPD：lymphomatoid granulomatosis, EBV ＋ immunodeficiency related LPD, chronic active EBV infection-B cell type, senile EBV ＋ LPD, etc. （2） EBV ＋ T/NK cell-LPD： CAEBV-T/NK cell type, hydroa vaccinifonne, hypersensitivity of mosquito bite, etc. In addition, EBV ＋ LPD is classified, based on the disease process, pathological and molecular data, as 3 grades： gradel, hyperplasia （polymorphic lesions with polyclonal cells）; grade 2, borderline （polymorphic lesions with clonality）; grade 3, neoplasm （monomorphic lesions with clonality）. There are overlaps between EBV ＋ LPD and typical hyperplasia, as well as EBV ＋ LPD and typical lymphomas. However, the most important tasks are clinical vigilance, early identification of potential severe complications, and treating the patients in a timely manner to avoid serious complications, as well as the active treatment to save lives when the complications happened.
作者
周小鸽
张燕林
谢建兰
黄雨华
郑媛媛
李文生
陈浩
刘芳
潘华雄
韦萍
王哲
胡余昌
杨开颜
肖华亮
吴梅娟
尹为华
梅开勇
陈刚
阎晓初
孟刚
徐钢
李娟
田素芳
朱军
宋玉琴
张伟京
Zhou Xiaoge Zhang Yanlin Xie Jianlan Huang Yuhua Zheng Yuanyuan Li Wensheng Chen Hao Liu Fang Pan Huaxiong Wei Ping Wang Zhe Hu Yuchang Yang Kaiyan Xiao Hualiang Wu Meijuan Yin Weihua Mei Kaiyong Chen Gang Yan Xiaochu Meng Gang Xu Gang Li Juan Tian Sufang Zhu Jun Song Yuqin Zhang Welting(Department of Pathology,Beijing Friendship Hospital Capital Medical University,Beijing 100050, China)
出处
《中华病理学杂志》
CAS
CSCD
北大核心
2016年第12期817-821,共5页
Chinese Journal of Pathology
